Osler-Weber-Rendu syndrome is a rare genetic disorder that commonly features high-flow arteriovenous malformations (AVM) within the pulmonary, intracranial, and visceral circulation. We present a patient with a unique case of OslerWeber-Rendu syndrome featuring a high-flow pelvic AVM in addition to fibromuscular dysplasia affecting multiple vascular beds. This required a unique modification of our embolic therapeutic approach for adequate treatment of the AVM. (J Vasc Surg Cases 2015;1:16-9.) Osler-Weber-Rendu (OWR) syndrome is a rare genetic disorder that commonly features high-flow arteriovenous malformations (AVM) within the pulmonary, intracranial, and visceral circulation. We present a patient with a unique case of OWR syndrome featuring a high-flow pelvic AVM in addition to fibromuscular dysplasia (FMD) affecting multiple vascular beds. Consent to publish this case report was obtained from patient.
CASE REPORTA 59-year-old woman with a history of OWR syndrome was evaluated in consultation for a symptomatic left pelvic AVM discovered on a computed tomography (CT) scan. Symptoms included a dull, throbbing ache, waxing and waning in severity, with exacerbation of symptoms after activity. She also reported a personal and first-degree relative history of chronic, persistent epistaxis. Her physical examination was remarkable for multiple, widespread erythematous macules (telangiectasias) throughout the volar aspect of the acral digits, tongue, and buccal mucosa (Fig 1, A and B). Left lower quadrant tenderness to deep palpation and a faint local bruit was appreciated.The CT scan confirmed the presence of large-caliber, tortuous arterial feeders supplying the nidus of a high-flow left pelvic AVM with prompt shunting into an aneurysmal draining vein, which eventually emptied into the left gonadal venous system (Fig 1, C). The arterial supply seemed to arise from the hypogastric or the gonadal arteries, or both. Results of the laboratory evaluation were unremarkable aside from mild microcytic anemia.The patient was offered a diagnostic angiography with the option for transcatheter embolization of this symptomatic, high-flow AVM.Technical details. The procedure was performed in a hybrid operating suite, with the patient under general anesthesia. No systemic anticoagulation was given; however, generous and frequent heparinized saline irrigation of sheaths and catheters was performed throughout the case. Flush aortography confirmed the presence of a high-flow AVM supplied by an enlarged, redundant, and extremely tortuous left gonadal artery, with prompt shunting into aneurysmal draining veins, which eventually emptied into the left gonadal venous system. No significant left renal vein hemodynamic abnormalities were noted. Selective catheterization of the left hypogastric artery revealed no association with the AVM. Also noted were diffuse, medial fibrodysplastic lesions with a characteristic chain of beads appearance involving the bilateral external iliac arteries and the left renal artery (Fig 2, A and B).Selective...